Related Experiment Video
Updated: Jul 8, 2026
08:41
Monitoring Endoplasmic Reticulum Calcium Homeostasis Using a Gaussia Luciferase SERCaMP
Published on: September 6, 2015
Diseases involving the Golgi calcium pump
J Vanoevelen1, L Dode, L Raeymaekers
1Laboratory of Physiology, KULeuven Campus Gasthuisberg O&N1, Herestraat 49 bus 802, B-3000 Leuven, Belgium.
Sub-Cellular Biochemistry
|January 16, 2008
Summary
Secretory-pathway Ca2(+)-transport ATPases (SPCA) are vital for Golgi apparatus function. Defects in the human SPCA1 gene (ATP2C1) lead to Hailey-Hailey disease, a skin disorder.
Area of Science:
- Cell Biology
- Biochemistry
- Genetics
Background:
- Secretory-pathway Ca2(+)-transport ATPases (SPCA) are essential for maintaining Golgi apparatus homeostasis.
- These ATPases facilitate the transport of Ca2+ and Mn2+ ions, crucial for numerous cellular processes within the Golgi.
Purpose of the Study:
- To review the fundamental properties and functional significance of SPCAs.
- To elucidate the connection between SPCA1 gene defects and the pathogenesis of Hailey-Hailey disease.
Main Methods:
- Literature review of existing research on SPCAs.
- Analysis of the role of the ATP2C1 gene in cellular function and disease.
Main Results:
- SPCA proteins are critical for intracellular calcium and manganese transport.
- Mutations in the ATP2C1 gene are directly linked to the development of Hailey-Hailey disease.
Conclusions:
- Understanding SPCA function is key to comprehending Golgi apparatus physiology.
- Targeting SPCA1 gene defects offers potential therapeutic avenues for Hailey-Hailey disease.
More Related Videos
13:40Live Cell Calcium Imaging Combined with siRNA Mediated Gene Silencing Identifies Ca2+ Leak Channels in the ER Membrane and their Regulatory Mechanisms
Published on: July 7, 2011
13:08Quantitative Localization of a Golgi Protein by Imaging Its Center of Fluorescence Mass
Published on: August 10, 2017
Related Concept Videos
01:33Overview of Secretory Vesicles
Secretory vesicles, also known as dense core vesicles (DCVs), are membrane-bound vesicles that transport secretory proteins, such as hormones or neurotransmitters. Regulated secretory vesicles transport proteins from the trans-Golgi network to the exterior of the cell. Proteins present in regulated secretory vesicles are required to be rapidly exocytosed in large amounts upon a specific stimulus.
Various proteins regulate the aggregation of molecules inside the secretory vesicles. Chromogranins...
Various proteins regulate the aggregation of molecules inside the secretory vesicles. Chromogranins...
01:12Golgi Matrix Proteins
Golgi matrix proteins are a group of highly dynamic proteins that maintain the stacked structure of Golgi. These proteins adapt to rapid morphological changes of the Golgi during the cell cycle. During cell division, mild proteolysis removes these connections resulting in Golgi unstacking. In The daughter cells, these proteins help reassemble the unstacked Golgi.
One of the first identified Golgi matrix proteins was GM130, a rod-like protein located in the cis-Golgi. Subsequently, many Golgi...
One of the first identified Golgi matrix proteins was GM130, a rod-like protein located in the cis-Golgi. Subsequently, many Golgi...
01:26Transport Across the Golgi
While it is unclear how molecules move between adjacent Golgi cisternae, it is apparent that the molecules move from cis- cisterna, the entry face, to the trans- cisterna, the exit face. Experiments initially suggested vesicles that bud from one cisterna and fuse with the next cisterna to transport proteins between the cisternae. This vesicular transport model describes the Golgi apparatus as a relatively static structure with a unique enzyme composition in each cisterna. Molecules are...
01:34ATP Driven Pumps II: P-type Pumps
The P-type pumps are a large family of integral membrane transporter ATPases. They are divided into five major types based on substrate specificity, from I to V.
A typical P-type pump has three cytosolic domains: nucleotide-binding (N), phosphorylation (P), and activator (A) domains. These domains are connected to the membrane-spanning helices by short amino acid segments. ATP hydrolysis and covalent phosphoenzyme intermediate formation are crucial parts of the catalytic cycle. At the highly...
A typical P-type pump has three cytosolic domains: nucleotide-binding (N), phosphorylation (P), and activator (A) domains. These domains are connected to the membrane-spanning helices by short amino acid segments. ATP hydrolysis and covalent phosphoenzyme intermediate formation are crucial parts of the catalytic cycle. At the highly...
01:22Lysosomal Hydrolases
Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
01:45Vesicular Tubular Clusters
After budding out from the ER membrane, some COPII vesicles lose their coat and fuse with one another to form larger vesicles and interconnected tubules called vesicular tubular clusters or VTCs. These clusters constitute a compartment at the ER-Golgi interface known as ERGIC (Endoplasmic Reticulum Golgi Intermediate Compartment). The ERGIC is a mobile membrane-bound cargo transport system that sorts proteins secreted from ER and delivers them to the Golgi.
With the help of motor proteins such...
With the help of motor proteins such...